Congenital Chylothorax: A Unique Presentation of Nonimmune Hydrops Fetalis in a Preterm Infant.

Background: Nonimmune hydrops fetalis secondary to congenital chylothorax (CC) is a rare disease process associated with high morbidity and mortality related to abnormal formation of the lymphatic system and disrupted management of fetal fluid. Hydrops fetalis is typically diagnosed prenatally by the presence of pleural effusions or other fluid collection on ultrasonography. Congenital chylothorax is diagnosed when the analysis of pleural fluid is deemed chylous. Neonatal presentation is often respiratory distress secondary to lung compression or pulmonary hypoplasia. Management ranges from supportive medical management such as high-frequency ventilation, chest drainage, and nutrition support, to controversial therapies such as octreotide administration and chemical pleurodesis, to surgical interventions such as thoracic duct ligation and mechanical pleurodesis.

Purpose: To discuss a range of management techniques and supportive therapies for hydrops fetalis and CC.

Methods/search Strategy: PubMed and CINAHL were searched using the terms "hydrops fetalis," "congenital," "chylothorax," "neonate," and "preterm." Twenty-two English language articles published within the last five years were identified and included to determine the current body of evidence surrounding treatment options for hydrops fetalis and CC.

Case Findings: In this case, a preterm male infant presented at birth with prenatally known bilateral pleural effusions and ascites, later diagnosed as nonimmune hydrops fetalis secondary to CC. A combination of supportive medical management, octreotide therapy, and surgical intervention effectively resolved this infant's chylothorax and hydrops.

Implications For Practice: Antenatal intervention may be appropriate for some infants, otherwise supportive medical management including respiratory and nutrition therapies should be prioritized for infants born with hydrops fetalis and CC.

Implications For Research: Nonimmune hydrops fetalis secondary to CC presents a complex challenge for neonatal providers, as no definitive treatment strategy currently exists. Further research is needed to determine the safety and efficacy of controversial therapies including octreotide.